Case Report Acute cauda equina syndrome secondary to lumbar chordoma: case report and literature review Mehala Tharmabala, MB BCh, BAO (NUI), LRCPI, LRCSI, Danielle LaBrash, Rani Kanthan, MBBS, MS, FRCS, FRCPC, FCAP, MEd* Department of Pathology and Laboratory Medicine, University of Saskatchewan, Royal University Hospital, 103, Hospital Drive, Saskatoon, S7N 0W8, Canada Received 22 December 2011; revised 29 March 2013; accepted 14 June 2013 Abstract BACKGROUND CONTEXT: Chordomas are rare tumors in the craniospinal axis arising from persistent notochordal rests commonly seen in the skull base, including the clivus and the sacrum. Chordomas in the mobile spine occur less commonly. To the best of our knowledge, the clinical presentation of acute cauda equina syndrome (CES) due to chordoma of the lumbar vertebra is not published in the English literature. PURPOSE: To describe an unusual cause of acute CES resulting from chordoma of the lumbar vertebra and discuss management dilemmas in this clinical context. STUDY DESIGN: Case report with review and discussion. METHODS: We report the case of a 75-year-old man who presented with acute CES that was clinically considered a metastasis from his previously documented carcinoma of the urinary bladder treated a year ago. Clinical, radiological, and histopathological features of the case and a review of chordomas in the lumbar vertebrae in adults in the published English literature are presented. RESULTS: He underwent urgent surgical decompression with laminectomy of L3/L4 and L4/L5 with debulking and open biopsy of the tissue mass. Histopathological examination of the tissue mass confirmed the unsuspected diagnosis of chordoma. The salient features of chordomas in the lumbar vertebrae published in the English literature over the last 22 years are summarized. The origin, clas- sification, clinical presentation, and management protocols for lumbar chordomas are also reviewed. CONCLUSIONS: The clinical presentation of acute CES as the first symptom of chordoma in the lumbar vertebrae is extremely rare. Preoperative tissue diagnosis of this uncommon pathology is usu- ally unavailable. In the face of acute CES, surgical decompression remains the primary goal of man- agement with a planned definitive second-stage curative surgical resection for chordoma. Crown Copyright Ó 2013 Published by Elsevier Inc. All rights reserved. Keywords: Acute cauda equina syndrome; Lumbar chordoma; Benign notochordal cell tumors; Malignant chordomas; Management dilemmas Background context Chordomas representing 1% to 4% of all primary bone tumors are rare, slow growing neoplasms arising from no- tochordal remnants in the cerebrospinal axis most com- monly seen in the skull base, including the clivus and the sacrum [1–16]. Chordomas are seen in approximately 15% of the mobile spine and approximately 33% to 40% of the mobile vertebral column chordomas are observed in the lumbar region [2,4,6–9,11,12,14,15,17–20]. Lumbar chordomas are slow growing tumors that usually present with chronic low back pain [2,9,12,17,19,21]. Acute cauda equina syndrome (CES) resulting from a chordoma of the lumbar vertebrae as a first symptom is an extremely un- usual clinical presentation. Purpose We report the case of a lumbar chordoma presenting with acute CES that was clinically considered to arise from FDA device/drug status: Not applicable. Author disclosures: MT: Nothing to disclose. DL: Nothing to disclose. RK: Nothing to disclose. * Corresponding author. Room 2868 G-Wing, Royal University Hospi- tal, 103 Hospital Drive, Saskatoon, SK Canada S7N 0W8. Tel.: (306) 655- 2158; fax: (306) 655-2223. E-mail address: [email protected](R. Kanthan) 1529-9430/$ - see front matter Crown Copyright Ó 2013 Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.spinee.2013.06.058 The Spine Journal 13 (2013) e35–e43
Transcript
The Spine Journal 13 (2013) e35–e43
Case Report
Acute cauda equina syndrome secondary to lumbar chordoma: case reportand literature review
Department of Pathology and Laboratory Medicine, University of Saskatchewan, Royal University Hospital, 103, Hospital Drive, Saskatoon,
S7N 0W8, Canada
Received 22 December 2011; revised 29 March 2013; accepted 14 June 2013
Abstract BACKGROUND CONTEXT: Chordomas ar
FDA device/drug
Author disclosures
RK: Nothing to disclo
* Corresponding a
tal, 103 Hospital Driv
2158; fax: (306) 655-
E-mail address: ra
1529-9430/$ - see fro
http://dx.doi.org/10.10
e rare tumors in the craniospinal axis arising frompersistent notochordal rests commonly seen in the skull base, including the clivus and the sacrum.Chordomas in the mobile spine occur less commonly. To the best of our knowledge, the clinicalpresentation of acute cauda equina syndrome (CES) due to chordoma of the lumbar vertebra isnot published in the English literature.PURPOSE: To describe an unusual cause of acute CES resulting from chordoma of the lumbarvertebra and discuss management dilemmas in this clinical context.STUDY DESIGN: Case report with review and discussion.METHODS: We report the case of a 75-year-old man who presented with acute CES that wasclinically considered a metastasis from his previously documented carcinoma of the urinary bladdertreated a year ago. Clinical, radiological, and histopathological features of the case and a review ofchordomas in the lumbar vertebrae in adults in the published English literature are presented.RESULTS: He underwent urgent surgical decompression with laminectomy of L3/L4 and L4/L5with debulking and open biopsy of the tissue mass. Histopathological examination of the tissue massconfirmed the unsuspected diagnosis of chordoma. The salient features of chordomas in the lumbarvertebrae published in the English literature over the last 22 years are summarized. The origin, clas-sification, clinical presentation, and management protocols for lumbar chordomas are also reviewed.CONCLUSIONS: The clinical presentation of acute CES as the first symptom of chordoma in thelumbar vertebrae is extremely rare. Preoperative tissue diagnosis of this uncommon pathology is usu-ally unavailable. In the face of acute CES, surgical decompression remains the primary goal of man-agement with a planned definitive second-stage curative surgical resection for chordoma. CrownCopyright � 2013 Published by Elsevier Inc. All rights reserved.
Chordomas representing 1% to 4% of all primary bonetumors are rare, slow growing neoplasms arising from no-tochordal remnants in the cerebrospinal axis most com-monly seen in the skull base, including the clivus and the
status: Not applicable.
: MT: Nothing to disclose. DL: Nothing to disclose.
nt matter Crown Copyright � 2013 Published by Elsevier
16/j.spinee.2013.06.058
sacrum [1–16]. Chordomas are seen in approximately15% of the mobile spine and approximately 33% to 40%of the mobile vertebral column chordomas are observedin the lumbar region [2,4,6–9,11,12,14,15,17–20]. Lumbarchordomas are slow growing tumors that usually presentwith chronic low back pain [2,9,12,17,19,21]. Acute caudaequina syndrome (CES) resulting from a chordoma of thelumbar vertebrae as a first symptom is an extremely un-usual clinical presentation.
Purpose
We report the case of a lumbar chordoma presentingwith acute CES that was clinically considered to arise from
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a spinal metastasis. Urgent surgical decompression with de-bulking and biopsy of the tumor mass revealed the unsus-pected diagnosis of chordoma arising in the lumbarvertebrae.
Methods
Case report
A 75-year-old man presented to the emergency witha 4-week history of severe low back and leg pain with in-creasing motor weakness and progressive bowel and blad-der dysfunction. He also admitted to a weight loss of 20lbs in 3 months. On physical examination, he had pain onmovement of the spine with bilateral Grade IV motorstrength in both lower extremities. There was numbnessin both feet and ankles with loss of ankle reflexes bilater-ally. There was a diminished right knee reflex and an absentleft knee reflex. His anal tone was decreased, and he hadsensory deficits in the saddle area. The clinical findingsconfirmed the diagnosis of acute incomplete CES. His sig-nificant medical history included bladder carcinoma (diag-nosed in 2009), Type 2 diabetes with peripheral neuropathy,coronary artery disease, myocardial infarction (in 1998),hypercholesterolemia, and essential hypertension.
Urgent investigations including computed tomographyand magnetic resonance imaging of the spine showed thepresence of a large, expansile mass centered in the L4 ver-tebral body with evidence of posterior cortical disruption
Fig. 1. Magnetic resonance imaging spine radiographs. A large expansible lesion
T1 dark, T2 bright and shows heterogeneous post-contrast enhancement. Severe st
also seen.
and extension of a lobulated soft-tissue mass into the epidu-ral space causing severe stenosis with compression of thedescending L4/L5 and the sacral roots posteriorly with nar-rowing of the thecal sac. This mass was intrinsically darkon T1 with hyperintense bright signals on T2 and showedheterogeneous post-contrast enhancement (Fig. 1). In viewof his medical history of bladder carcinoma diagnosed in2009, the preoperative working diagnosis of this masswas that of spinal metastasis.
As acute incomplete CES is a spinal emergency requir-ing urgent decompression surgery, he was prepared for sur-gical intervention. L3–L5 posterior decompression wascarried out with laminectomy bilaterally at L3–L4 andL4–L5. Decompression was carried to the edge of the the-cal sac to identify the tumor that was pressing on the exit-ing nerve roots posteriorly. The tumor was debulked anda tissue biopsy of the mass arising from the L4 vertebralbody was submitted for histopathological examination.Postoperatively, the patient was able to weight bear, andthere were no immediate or delayed complications.
On histopathological examination, the gross specimenweighing 1.4 g consisted of ragged pieces of pale-tan,slightly gelatinous material admixed with blood clot. Micro-scopic examination of the formalin fixed paraffin embeddedspecimen showed the presence of a lobulated neoplasm ina prominent extracellular myxoid matrix. The neoplasticcells were composed of syncytial cords of ‘‘physaliphorouscells’’ that contained abundant pale septated and vacuolatedclear to bubbly cytoplasm with mild nuclear pleomorphism(Fig. 2, Left, Middle). Overt mitosis was not identified. Mild
is seen centered in the L4 vertebral body (white arrow). This is intrinsically
enosis with compression of the descending L4, L5, and sacral nerve roots is
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cytological atypia was also observed. Immunohistochemicalexamination showed that the lesional cells stained negativefor glial fibrillary acid protein and were strongly positiveto antibodies with low-molecular weight keratin, CK19, Ep-ithelial Membrane Antigen, and vimentin (Fig. 2, Right).The coexpression of epithelial and mesenchymal immuno-histochemical markers supported the notochordal lineageof the lesional cells. Ki67 was moderately expressed in thelesional cells. The above histomorphological features inconjunction with the immune-phenotype observed con-firmed the diagnosis of chordoma.
At discharge, despite lengthy discussions, the patient re-fused any further surgery. The concept of curative surgeryas an elective planned second-stage procedure that may in-volve a combined anterior and posterior approach withwide excision for tumor bed clearance and the role of radio-therapy will be re-explored for discussion at his next clinicvisit.
Literature review
A literature search limited to the English language wasperformed using the search terms ‘‘chordoma,’’ ‘‘lumbarvertebrae,’’ ‘‘spinal cord,’’ ‘‘vertebral chordoma,’’ ‘‘chordo-ma of mobile spine,’’ ‘‘spinal cord compression,’’ and ‘‘man-agement.’’ Research engines used included EMBASE:Excerpta Medica & EMBASE Classic, National Library ofMedicine interface PubMed, Medline, and Scopus. Pub-Med’s ‘‘MeSH (Medical Subject Headings)’’ feature wasused to further extend the search and obtain articles fromtheir bibliographies and secondary resources that were iden-tified and retrieved for review.
Results and discussion
Chordomas are rare, slow-growing, low-grade tumorswith an incidence of 0.5 cases per million or 0.1 new casesper 100,000 per year range [2,5,16,19–24]. Despite theirrarity (1%–4%), chordomas are the most common primarymalignant bone tumor in both the sacrum and the mobilespine (also known as the true vertebral chordoma)[16,24]. Vertebral chordomas are most commonly found
Fig. 2. Photomicrographs of the vertebral lesion. (Left) Haematoxylin- and eos
a neoplasm composed of cords of cells (*) in a myxoid background. (Middle) H
shows the presence of ‘‘physaliphorous cells’’ with abundant pale clear to
Immunohistochemical-stained slides with low-molecular weight keratin, CK19, an
in the cervical spine (nearly 50%) and less commonly inthe lumbar (40%) spine and the thoracic spine [2,24]. Lum-bar chordomas are rare and account for approximately 6%of all spinal chordomas [2]. Chordomas most commonlyoccur in adults and the elderly, although they can occurat any age with rare cases reported in children [9,23,24].Although, the median age of diagnosis is 60 years, vertebralchordoma tend to occur in the younger age group [8,22,23].Chordoma is far more commonly seen in men comparedwith women with a ratio of 2:1 [2,4–6,8,16,19,24].
Chordomas are believed to arise from primitive noto-chordal remnants (ecchordosis physaliphora) that run theentire length of the spine [1,2,4–10,12–26]. The primitivenotochord, a precursor to the human spine, is an embryonicstructure composed of cells derived from the mesodermthat undergoes significant regression by birth and completeinvolution by the end of the first decade of life [27]. Theprimitive notochord typically disappears during the seventhweek of gestation, but persistent remnants can still be foundin adults in the center of the nucleus pulposus of the inter-vertebral discs [2,18,28]. The nucleus pulposus is the onlypersistent adult derivative of the notochord. Chordomas,however, are not seen to originate from the nucleus pulpo-sus structure but instead arise within the vertebral body, andtherefore, they are believed to arise from the non-neoplasticremnants of the notochord ecchordosis physaliphora, whichare believed to exist in the vertebral body [2,29]. Thus,chordomas belong to the dysontogenetic bone tumors[30]. Consequently, chordomas are limited to the axial skel-eton predominately occurring in the midline involving theskull base, mobile spine, or sacrum.
Ectopic notochordal rests in adults was first described in1865 by Luschka who found them on the clivus at the baseof the skull and were subsequently termed ‘‘ecchordrosisphysaliphora spheno-occipitalis’’ [29]. It was consideredas one of the precursor lesions of classic chordomas andwas found in 0.6% to 2.0% of autopsy cases [31,32]. Thefirst reported case of the benign chordal tissue occurringwithin the vertebral body was by Ulich and Mirra in 1982and was labeled as ‘‘ecchordosis physaliphora vertebralis’’[28,29]. Benign notochordal cell tumors, a recently discov-ered distinctive intraosseous lesion of notochordal origin,have been hypothesized to have a relationship with
in-stained slides at low power (lens objective �2) shows the presence of
aematoxylin- and eosin-stained slides at high power (lens objective �10)
bubbly cytoplasm (black arrow) and eccentric small nuclei. (Right)
d vimentin are strongly positive (brown staining cells) in the lesional cells.
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chordomas and may even be precursors to chordomas. Inthis context, we concur with previous authors of a spectrumof notochordal-derived lesions ranging from benign to ma-lignant as listed in Fig. 3 with many cases confirming thepresence of classic chordoma arising from an intraosseousbenign notochordal cell tumor [32,33]. Benign chordomasinclude intraosseous notochordal tumors, giant notchordalvertebral tumor, giant notochordal rest, and giant notocho-chordal harmatomas [27,28,31–35]. The distinction be-tween benign and malignant chordoma is clinicallyimportant because the management and overall prognosisare significantly different. Benign chordomas do not requiresurgical management (careful clinical and radiologicalfollow-up is recommended) unless they undergo malignanttransformation [32,34].
Three variants of malignant chordoma are identified onhistopathological examination. These include the classicchordoma, the chondroid chordoma, and the dedifferenti-ated (sarcomatous) chordoma (Fig. 3). Classic chordomasconsists of lobulated growth pattern composed of roundto polyhedral epitheliod cells in cords or clusters with vac-uolated bubbly cytoplasm (physaliphorous cells) enmeshedin a myxoid matrix with rare mitosis as seen in the indexedcase. Occasionally, classic chordomas may contain focalareas with spindle cell morphology suggesting sarcomatoid,or marked epithelial appearance (carcinoma-like chordo-ma), or signet ring cell formation. Such histological vari-ants continue to be diagnosed as classic chordoma,provided they remain mitotically inactive. Chondroid chor-doma with areas resembling hyaline cartilage in addition tothe conventional classic chordoma comprises approxi-mately 15% of all chordomas and is most commonly
Fig. 3. Pathologic classification of chordomas. This figure represents a flow c
chordomas.
reported in the craniocervical junction. Very rarely, histo-pathological examination shows the presence of an aggres-sive, dedifferentiated, or ‘‘sarcomatous’’ (5%), high-gradevariety of chordoma that may show areas resembling eitherfibrosarcoma, malignant fibrous histiocytoma, or osteosar-coma adjacent to the conventional classic chordoma[14,16,23]. The most frequent reported location for dedif-ferentiated chordoma is in the sacrum but rare cases havealso been reported at cranial, vertebral, and even mediasti-nal sites [16].
The clinical presentation of a vertebral chordoma isclosely related to the location of the tumor and the tumor’scompression of the root, spinal cord, and paravertebral tis-sues. The mean duration of symptoms was 22 months in thestudy by Cheng et al. (range, 0.7–120 months) [3]. Pares-thesia and pain are the most common presenting symptomsof lumbar chordomas and generally precede other symp-toms [2,21]. Nocturnal back pain not responding to bed restis also common [2,12]. Lumbar chordomas are easily con-fused with less rare tumors such as aneurysmal bone cyst,giant cell tumor, hemangioma, myeloma, and metastasesleading to difficulty with accurate preoperative diagnosis[2,12,21]. The clinical presentation of acute CES as the firstsymptom of chordoma in the lumbar vertebra has not beenpreviously described.
Acute CES is a relatively uncommon condition and isone of the few known spinal surgical emergencies [36].Cauda equina syndrome occurs because of compressionof the nerve roots of the cauda equina with resulting dys-function of the sacral and lumbar nerve roots [37]. Themain clinical manifestation of CES includes bilateral sad-dle sensory deficit with bowel, bladder, and sexual
hart delineating the histopathological varieties of benign and malignant
Table
Chordoma in the lumbar vertebra in adults as reported in the English literature (PubMed, Medline, Scopus, Embase, Google/Google Scholar)—search terms included ‘‘vertebral chordoma’’ AND ‘‘chordoma
of mobile spine’’ AND ‘‘lumbar chordoma’’
References
Year
reported
No. of cases
mobile spine
No. of cases
lumbar spine Gender Age (y) Location Symptoms Management Outcome
M, male; F, female; LBP, low back pain; LP, leg pain; KP, knee pain; PAA, posterior-anterior approach; NA, not available; RTR, radiotherapy; NED, no evidence of disease; IL, intralesional; MR, marginal
resection; DWD, died with disease; AWD, alive with disease; VB, vertebrectomy; APA, anterior-posterior approach; DOC, died of other causes; EB, en bloc (wide); P, palliative.
* These series include chordomas in kids and adults. Case-specific details regarding lumbar vertebral in adults are not available in these reports.
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dysfunction [36,37]. In this context, CES is clinically cate-gorized as incomplete CES and complete CES. The latterrefers to complete paralysis of the bladder leading to estab-lished urinary retention with overflow urinary incontinencein contrast to the former with milder neurologic deficits andpredominately still normal functioning bladder [36]. Themost common cause of CES is a large central lumbar discherniation at the L4–L5/L5–S1 regions [36,37]. Less com-mon causes include spinal injury, post-manipulation, spinalanesthesia, space occupying lesions such as hematoma, ab-scess, schawnommas, ependymomas, lipomas, teratomas,arteriovenous malformations, metastatic tumors, ischemicinsults, and inflammatory conditions of the cauda equinaand spinal cord [36–39]. Acute CES as a result of chordo-ma in the lumbar vertebrae as seen in the indexed case hasnot been previously described. Patients with acute caudaequina compression especially those without urinary reten-tion (incomplete CES) require urgent decompression sur-gery [37,38,40]. The only debate that exists in thepublished literature is the timing of this surgery in relationto the influence of the outcome. The consensus of opinionis that decompression achieved within 48 hours of CESachieves better outcomes than delayed decompression be-yond 48 hours [37,40]. Thus, in our case, the primary goalof management was surgical decompression for acute in-complete CES. Following the confirmed postoperativediagnosis of a primary chordoma, this now creates di-lemmas regarding further management as there is potentialwide spread contamination of the tumor bed. This will ne-cessitate careful planning for second-stage definitive cura-tive resection of this potentially low-grade malignantneoplasm.
Chordoma is one of the most challenging mesenchymaltumors to treat effectively despite the relative slow growthrate of this tumor [41]. Surgery remains the primary treat-ment modality with radical resection as the accepted stan-dard therapy [1,9,24,42]. Adequate wide surgery with clearsafe margins is the primary goal of surgery. The achievementof negative surgical margins is however difficult in somecases due to the location of the tumor and in the index caseis fraught with difficulty due to the extensive ‘‘potential’’contamination of the tumor bed with unintentional tumorseeding in the operative field [1,24]. In such cases, insteadof wide margins requiring a major en-bloc resection, an in-tralesional margin is a secondary alternative [1,9]. However,local recurrences in such cases remain high [1,42]. In thiscontext, postoperative adjuvant radiotherapy is used forloco-regional control of the disease [1,24]. Although chor-domas are traditionally not radiosensitive, recent advancesin radiotherapy such as proton beam irradiation, the use ofhadron (high-dose protons or charge particles such as carbonions, helium, or neon) given alone or in combination havebeen used to improve the radiological and biological effect[1,23,41,43]. Combination therapy of photon irradiationtogether with razoxane has also been shown to be more ef-fective than photon irradiation alone [44]. In some cases,
a near complete pathologic response of chordoma to high-dose single fraction radiotherapy has been reported [45].Table summarizes chordomas in the lumbar vertebra inadults as reported in the English literature with special refer-ence to gender, age, location, symptoms, management, andoutcome [1–17,19–22,25,26,29,33,42,43,45–58].
Although classical chordomas have a very low sensitivityto chemotherapy, high-grade de-differentiated chordomashave been reported to respond to aggressive chemotherapy[1,59]. The era of targeted therapy has opened new perspec-tives for management in this rare chemo-radio resistantdisease. Imatinib mesylate targeting platelet-derived growthfactor receptors have been used successfully in patients withlocally advanced and/or metastatic disease [23,24]. Othermolecular-targeted agents such as cetuximab and gefitinibhave shown promising responses in some metastatic/recur-rent chordomas. Today, a multidisciplinary team approachwith surgeons, medical oncologists, radiotherapists, pathol-ogists, and radiologists is recommended for the managementof chordomas as loco-regional control with long-term sur-vival remains the primary goal [1].
Chordomas are very slow-growing tumors that are un-likely to metastasize early. The likelihood of metastasis isrelated to the primary location of the tumor. Metastasis isvery rare among intracranial chordomas (only 9%), morecommon for sacrococcygeal chordomas and most commonfor lumbar chordomas [2]. When metastasis does occur, thelung or lymph nodes are commonly involved followed bythe liver, musculoskeletal system, skin, brain, and/or otherinternal organs [1,2,4]. There is no direct relationship be-tween metastasis and survival rates [2]. Despite the slowrate of growth of chordomas, the most common cause ofdeath is due to local aggressiveness with multiple recur-rences [2,4]. The extent of surgical resection, that is, ‘‘mar-gin status’’ plays a major role in determining the length ofdisease-free-survival and overall long-term survival. Thelocation of the chordoma has also been shown to have aneffect on the survival rate with longer survival rates for spi-nal chordomas [2]. Stage is usually not a discriminatingfeature to determine outcome as most chordomas are lowgrade with cortical extension and rarely have metastases.Other predictive indicators that are found to be more usefulinclude the most proximal extent of tumor according to thevertebral level and the size of the tumor that seem to bestrong predictor for relapses [3]. The reported 5-year sur-vival rates for vertebral chordomas are approximately50%, and with effective surgical treatment, the disease-free interval at 5-years is 30% to 50% [2]. In summary,the survival rates for all chordomas worsen over time andonly a small minority of patients are truly cured.
Conclusion
In conclusion, to the best of our knowledge, this is theonly reported case of the clinical presentation of acute
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CES as the first symptom of chordoma in the lumbar verte-brae. Acute CES is a spinal surgical emergency requiringurgent decompression.
The slow rate of growth of chordoma associated witha low metastatic potential makes surgery the ideal first lineof treatment for this rare primary bone tumor. Although po-tentially curative, a margin-free en-bloc resection is oftenvery hard to achieve practically. Moreover, such surgeryshould not be performed without a presurgical tissue-specific histologic diagnosis and without technical exper-tise to perform a wide resection. The status of clear marginsis a very important prognostic/predictive factor being wellcorrelated with the incidence of local recurrences and over-all survival. In the given indexed case, surgical decompres-sion remains the primary goal of management witha planned definitive second-stage curative surgical resec-tion of the chordoma.
